A New Chapter

Well howdy folks. It's been some time since my last blog and consequently there is a lot of catching up to do. Emri's spinal rod lengthening surgery is quickly approaching. We now have less than a month. We head out on the 14th of September, and surgery is on the 17th. My heart sinks lower each day we get closer. I know summer is coming to a close...which is sad...but I also have been looking forward to just getting this next surgery behind us. PLEASE pray for a healthy and quick surgery/recovery for our sweet girl. 

We had been struggling to come up with a solution of who could watch Abe in the mornings while we were gone, since Brett's mom teaches mornings, and my mom teaches all day. Our prayers were answered when Brett's Aunt Ruth volunteered to fly here from New Mexico and help with Abe...what a relief! Abe knows Aunt Ruth and even gave her a few snuggles when she visited a few weeks ago. It feels so good knowing that he will be adored and spoiled by people he knows and loves while we are gone. This will be our longest time away from him, which will be hard...but less hard knowing he is being so well taken care of.

The title of this post is "A New Chapter." Our family has been going through an overload of change recently. To make an insanely long story very short...Brett now has his own company and has been working from home for a month. Over the past few months, God...in an amazing number of ways...has been making it completely clear that this was the path our family should be taking. Brett is enjoying his time working from home and loves the new flexability that comes with owning his own company. The name of his company will be ANC Architecture...although it will be ANC design until he officially gets his license. ANC stands for...you guessed it...A New Chapter! A new chapter for us and a new chapter for his clients. 

And because we are crazy people who love to make life overly stressful and dramatic (NOT) we will also be leaving Lynden sometime in the next while and moving to Mount Vernon. We will be staying there for the rest of our lives and never, EVER moving again..at least that's my vote!! 8 moves in 6 years is a little much for me. More details to come on that whole situation at a later time. 

This summer has been a good one. Emri turned FOUR on June 8. Every birthday we celebrate with Emri is filled with thankfulness and awe. We are so thankful that God has given us 4 amazing years with our incredible little girl and in awe of how far she has come in her short 4 years of life.  Emri got new leg/feet braces and is walking SO much straighter! It has been amazing to watch her improve every day. She absorbs information like a sponge and can't wait to start school again in a couple weeks. Abe is talking more, loving more, helping more, hurting more, and GROWING more. He has now reached the perfect ram-his-head-into-the-island-counter height, and seems to have inherited my desire to move walls/doorways when he walks. He loves his sister (although you wouldn't always guess it) and likes to copy her movements, phrases, and facial expressions. My kids are such a blessing to me. I love them more than I could ever explain.

 We had the opportunity to visit with all of the Van Andel relatives at a reunion in Michigan this summer. Although we discovered the horrifying result of combining Abe with the confined space of an airplane, the torture of the flight was worth the time with family. It was a special time with family and won't be soon forgotten.

Well the time has come--Abe's awake and break-time is over. Thanks for reading!

So here we are....another day of bad news. Emri had a sleep study done last week and I got the call today that the results were terrible. 8 apnea episodes an hour??!? 8?!? How have I not caught on to this? There have been plenty of nights that I have snuggled up to my little girl and listened for signs of apnea. Her O2 is dropping at night...how scary. She moves her legs a lot...why?? The pulmonologist that has read all of Emri's sleep studies wants her on oxygen at night immediately. We have to go the the ENT at Childrens and stick that dang scope up Emri's nose and down her throat...again. And we need more blood draws. I SO did not want to hear all of this.
I want to take a second to be honest...because being honest is only fair. It's only fair to anyone who reads this blog to get the real me. 
I just read a blog post highlighting special needs moms. It talked about how so many people respond to others' difficult situations with, "I don't know how you do it." Here's the thing....in my situation with Emri, what IS the proper way to "do it"??? To be frank, I have spent my fair share of time screaming, crying, and begging God for mercy. Even the moments where there isn't any major surgery or big echo appointment going on, I still get overwhelmed...I feel like I am constantly attempting to keep my head above water. Just yesterday I parked to pick Emri up from school and saw her class walking back from the playground. My eyes scanned the group for my tall, skinny girl...but then I saw her. She was the one being wheeled back to the classroom in the stroller. It might not seem like a big deal...but my head was spinning and my heart hurt. Did she feel sad that she couldn't walk all the way to the playground?? Was she looking at the kids walking in front of her and wondering why she wasn't with them?? Did she feel left out? The emotional part of me is drained. Always. 
I think about the verses in the Bible that talk about being anxious and I wonder, "God, HOW??" How am I supposed to sleep now...knowing that Emri quits breathing 8 times an hour every night??? I lay next to Emri and I can hear her extremely loud heart murmur...a constant reminder that her heart isn't healthy. Her heart is working so hard to counteract her leaky valves. It makes me feel nauseous. I have forgotten to give Emri her medicine a few times in the past two weeks. What mom just forgets to give her daughter life saving heart medicine??!? The guilt I feel every time I forget is severely overwhelming. 
So "how do I do it?"  I don't have a choice. I "do it" but I never feel like I am "doing it" well. I fail Emri, I fail myself, I fail God. How do I cope??? I cry, I talk things out, I write, and I PRAY. I am always praying for peace...for strength...that I am able to raise Emri to be strong....when I feel so weak. 
Please pray for us. Pray that tonight when we put that dreaded nasal canula in Emri's nose that she somehow musters the strength to accept it and fall asleep. Pray for healing...maybe the next sleep study will be better. Pray for true joy for our family...not just shallow happiness...but joy in the Lord. Pray for Brett and I...we love each other so much....but having a constantly drained wife can't always be fun. Pray for energy and strength for me to be the mom that God wants me to be for Emri and Abe. 
There will be a day with no more pain or tears...we know this and cling to it. But right now there is so much pain, and way too many tears. 

6 months in one post!!

Well regardless of the fact that our life has been moving at record pace for the past 6 months, this blog has most definitely seen better days. My apologies, dear blog, for leaving you in the dark for such a vast expanse of time.

Thanks to my frequent flying on facebook, I believe I will be able to jog my memory enough to summarize the last half year. Here we go.

A few big events worth mentioning: 

-Abe survived a fall down the stairs. He immediately attempted to do it again.

-Emri, (with no reasonable explanation with the exception of answer to prayer) started eating primarily by mouth instead of through her g-tube.
-Abe turned 1 on October 29....he had his cake, smashed it all over the place, and ate it too. SO hard to believe our little "baby Abee" is no longer a baby!

-Found out that Abe, at 1, weighed 3 pounds more than his 3 year-old sister. 

-Brett turned 29!! 

-My sister and her wonderful man were married on November 30. Emri was the flower girl and WALKED tall and proud down the isle. It was, of course, an incredibly touching moment for our family. Abe was the ring bearer...he only dropped the box once and stopped mid-isle to visit his grandpa. It was a very special day that will not be soon forgotten.  

-Abe survived a fall out of a home depot cart...onto cement. Worst. Mom. Ever.

-WE MOVED INTO OUR HOUSE! December 6 was the day we moved. It was extraordinarily stressful and incomprehensibly blissful all at the same time. Our upstairs remains, to this very day, a "junk zone", but once in a while we have ambitious thoughts about going up there to organize it all. Oh. And the slide is awesome.
-We got a puppy named Jack! Long story short...we have always wanted our kids to grow up with a dog. We wanted him to be well trained-so I took him to "grad school." It was a little embarrassing...but he graduated. We love him dearly...but he is still a work in progress.

-December 20-We got SNOW! Emri wanted to go outside and build sand castles. 

-December 24-We had our first official Christmas with our little family of 4. Last Christmas was spent at Johns Hopkins Hospital with Emri, who was recovering from her spinal rod surgery. We treasured the time spent together. 

-December 25-Brett's two brothers and their families flew here from Denver! We had a great time celebrating Christmas together and Emri LOVED the time spent with cousins. It was a special time for the Van Andel family!

-Emri had her very FIRST day of school on January 13th!! She loved it then and continues to love it now! She jolts out of bed to get ready for school and, without fail, has a giant grin on her face when I go to pick her up. She has a personal aid to help with her stability, which puts my heart at ease dropping her off. 

-I turned 26. Bring it on!

-Found Abe in the dryer, laughing hysterically.

-We watched the Seahawks WIN the SUPERBOWL.

-We spent a night at Children's in early February due to Emri getting so sick with a virus. She did great and recovered quickly after. Emri got an echo while we were at Children's showing no additional growth of the aorta and the same amount of mitral valve leakage as the last echo. Thankful for this!!

-February 23 was the first day of our Lynden snowstorm!! We had a great three days watching it pour snow. We got like 2 feet! The kids made it out once...for a total of 3 minutes.

-Abe achieved "double ear infection" status!
-Emri and I had the privilege of speaking to a group of nursing students at Skagit Valley College about marfan syndrome! Emri got super spoiled with a TON of doc McStuffins gear...including her very own doctors coat. 

-Abe rammed the side of his eye on a window ledge (all according to Emri as the sole witness) and gave himself a nice black eye.

So there you have it! 6 months summed up into 20 bullet points. Despite acquiring plenty of pesky viruses this winter, Emri has stayed away from the hospital all but once...which we have been so thankful for. She continues to blow us away with her strength and her gigantic personality...she just loves life and doesn't stop singing about it! I have determined that Abe wakes up every morning thinking, "how am I going to give my mom a heart attack today???" He is our little stunt man, continuously looking for new ways to injure himself.

We love our God and continue to strive to serve Him in all that we do, say and think. Our latest memory verse that we have been working on with Emri has been Psalm 19:14.

"Let the words of my mouth and the meditations of my heart be acceptable in Your sight, O Lord, my Rock and my Redeemer." 

Always a good verse to have fresh in your head. 

And now, it's time for bed. It was nice writing a blog that contained no surgery drama and only one hospital stay! Praise the Lord for the health and strength that He has given us over the past 6 months! Thanks for reading!

First Rod Extension, October 1, 2013

For those of you who check this blog, but do not have access to facebook---here is what has been going on here, in Baltimore, over the weekend and today. 

Friday, September 27:

So today was a day of walking, eating, and exploring Baltimore...for the 4th time! We actually feel like we know where we are going now! We also have the TWO (!!!?!?) starbucks locations memorized:) Yesterday we met with Pre-op in the morning, had Emri's labs taken, and met with a pulmonary Dr. in the afternoon. Everything went relatively well-they even got Emri's vein the FIRST time (we made sure to request the lady that we had success with last time). Pre-op is always the scariest because of all the risks discussed and we always can sense how nervous they are about working with our fragile, complicated Emri. We are now trying to figure out when we should be giving Emri her medications before surgery-because they affect blood pressure. During Emri's g-tube surgery, her blood pressure dropped suddenly and dramatically---which they seem to attribute to her meds....so we are sorting all of that out, trying to get her on a good medication schedule. Tomorrow we hope to get some free passes to the zoo from the Ronald McDonald house where we are staying. Emri has been wanting to see elephants:) Monday we have an echo, see Dr. Dietz and his genetic counselors, and meet with Emri's surgeon, Dr. Sponseller. Tuesday, at 5:30 AM we head in for surgery. So, all of you West coasters can think of us at 2:30 AM your time...just kidding! For those who were asking, our address here is 

635 West Lexington Street
Baltimore, MD 21201 Room number 217

Monday, September 28:
"So, I'm not going to lie...that was a. long.day. Got up at 6, on the shuttle by 7, and pre-op with Sponseller at 8. Of course we gave ourselves 2 seconds to grab COFFEE before our appointment. It's funny how much less dependent everyone here is on coffee....what is the deal??!? Anyhow, Appointment with Sponseller went fine. Nothing surprising. Sponseller is optimistic about the surgery and feels confident that Emri's recovery will be way less intense than the initial rod placement surgery. We DID learn, however, that Emri will eventually need a new set of rods as she grows...which is slightly disappointing. Next we had Emri's eye appointment which was nothing less than completely frustrating and almost entirely fruitless. They tried to perform a number of tests on Emri to get glaucoma readings, eye length...etc... It wasn't so much that Emri was uncooperative, but more the eye technicians thinking that, for some reason, a 3 year old has the capability of sitting COM-PLETE-LY still for 5 years while they get some dot inside of another dot which has to line up with a 3rd dot...or something to that effect. Eventually it was decided that they would just get the readings they needed while Emri was put out tomorrow morning...which is what I thought the plan was in the first place...uhg. THREE HOURS later we were headed for Emri's echo. Emri fell asleep during her echo, which was a good little break for her. The good news is that her heart looks great! Stable from the last echo, and ready for surgery. The only slightly bad news is that they did find a small hole, or flap between ventricles. This is actually relatively common...25% of us have it. It is even more common in marfan syndrome. This hole can raise the chances of stroke or pulmonary blood clot...but it happens very rarely. We also need to always make sure that when Emri gets an IV that the nurse has the bubble blocker hooked up with the IV. So not SUCH a big deal....but still...just ONE more thing to add to the list. If and when Emri would ever need a heart surgery, they will stitch this hole shut.

We got on the shuttle about an hour ago and are finally back in our room at the Ronald McDonald house. As I was sitting on the shuttle feeling overwhelmed, exhausted and wondering where I was going to get the strength to fight through this next week, the song, "I Can Only Imagine" came on the radio (our driver is awesome). Thanks to our wonderful God for the gentle, yet blatant reminder of who is in control, and what we are living for. What a crazy day...but what an AMAZING God we serve."




Tuesday, Ocober 1, Surgery Day:
7:30AM: "Emri fought hard to stay awake. It is the most unnatural thing as a mother to sit there, unable to do anything, while your baby is in that much distress...and then leave her in the hands of a team of people you just met 10 minutes ago. God take care of my beautiful, brave girl."

9:00AM: "Surgery is finished! Emri did well. Praising God for this news. They are finishing up with some eye tests, and will hopefully get her off the vent soon as well. Can't wait to see her and start talking about all of the things we get to look forward to now that this is over with. Thank you for all of the prayers, sweet messages, and kind words. I will update when we are settled in the PICU."

1:00PM: "So thankful to see Emri breathing on her own and waking up...but it is nothing less than torturous watching her go through this all again. She is just so agitated and in pain. So far she has said ,"I don't like this," and keeps asking if , "this will be done soon."

5:30PM: "Emri is doing well stat wise. Oxygen is on low and bp and heart rate have been stable. She has said, probably 50 times, that her stomach, chest, and back hurt. It has now turned into , "mommy, all of those 'fings' still hurt." She is being so brave. Praying for relief for my sweet girl."


Wednesday, October 2:
9:00AM: "We just got news that Emri will most likely be moving to "the floor" today already! Her stats have all been good-it's just pain management and getting her to move around a little. The picture below was last night when Emri said she wanted to cuddle with me (melt my heart). We got all settled and then she started crying and said "I can't do this" (break my heart). She is busy right now watching Doc McStuffins and fading in and out of morphine induced sleep. She hasn't had a wet diaper in a while so we are hoping we don't have to catheterize her again. We are praying for less pain and more pee!!"




10:00PM: "Our little punkin had a pretty intense pain attack about 2 hours ago and is now on vallium along with her morphine. She also has a 101 degree fever. She has had her arms wrapped around my neck the whole time. She is a little more comfy now and watching mickey mouse. Never fun to feel like we are taking one step forward and two steps back."

Thursday, October 3:
10:00AM: "Emri is doing better this morning. Fever is gone. Lung x-ray came back looking pretty good-just a teeny tiny collapse in the upper left lung- but nothing to do anything about. The biggest obstacle now is getting Emri's digestive system moving again. Vallium and morphine have slowed it down, so we are waiting for the pain team to come and switch her to some more digestive system friendly meds."

3:00PM: "Look who's sitting up and SMILING??!? Feeling a little better! AND she gets to wear her new pink JH shirt!!""


Friday, October 4:
11:00AM: "Emri was having SUCH a great night last night until about 7 or 8 and then things got sticky again. She came down with a fever that tylenol didn't seem to affect, her stomach became (and remains) super distended and she is in a good amount of pain again. She is now being tested for a few things to make sure her digestive system is healthy. We got to ride a wheelchair around the hospital to get an x-ray and Emri is seeming slightly more comfortable than last night. It's frustrating to watch her go from happy and comfortable to sick and in pain. Hoping things go up from here."

Saturday, October 5:
10:00AM: "My puffy little sweetheart. Stomach still hurting, retaining too much fluid, not eating, and building towers out of syrup is what's going on this morning."

Sunday, October 6:
10:00AM: "Absolutely feeling discouraged this morning. Emri's oxygen dipped into the 80's quite a few times last night. She is now on oxygen and received an x-ray this morning to check the lungs. Pretty confident there is a partial collapse. She is more puffy than she has ever been, in a substantial amount of pain, and her IV just slipped out of her foot...so she has to be re-stuck. My poor, sweet girl is quite miserable and it is so hard to watch. Please pray for healing for Emri, and patience for us."
Monday, October 7:
9:00AM: "Thank you to everyone for the prayers, encouragement and support. Emri FINALLY got some relief from her stomach issues last night following a few painful, uncomfortable treatment methods. Her stomach is slowly deflating and looking closer to normal size. She does have a small lung collapse which we will start treating more aggressively today with nebulizers and chest PT. Our goal today is to be up and moving as much as possible and to get her feeds closer to what she was on at home. She is the bravest, sweetest, and toughest kid."






1:00PM: "We got to visit with this cutie and her mom and dad today!! How refreshing to get to see them and talk with friends who have been through so many of the same things. We love the Jennings family!! Thank you for the visit and all of the sweet presents and cookies!!" 

 

Tuesday, October 8:

 

10:00AM: "So we are being discharged this morning. I am excited to get outa here, but a little nervous because of where Emri is at from a recovery standpoint. We have an appointment with Dietz at noon and a pulmonary appointment at 3 today. We are set to take off back to Washington tomorrow morning. We are happy to be at this point and thanking God for his healing this far...but we are going to have to be c...autious and in tune with how Emri's body reacts to the next few days. Its always a little nerve wracking leaving the nurses here who take such good care of Emri and who watch every heart beat and breath on her monitor. Thank you for all of the heartfelt prayers and words of encouragement, and please join us in continuing to pray for our sweet girls healing."

 

5:00PM: "Praise GOD for two great appointments today!! Cardio with Dietz was terrific...have we mentioned that we LOVE him??!? We always walk out of our meetings with him feeling like we can fly. The pulmonary appointment was also encouraging and we... feel like Emri is in the best hands possible. We weighed Emri before her appointments and found out she has an extra 9 POUNDS of fluid on her body right now...9 POUNDS! It was quite something to see the number 33 on that scale! We are hoping moving around and getting some more protein in her body will help with that and the stomach distension. Emri is SO SO happy to be out of the hospital. When we left the room this morning she couldn't stop talking about how excited she was. We asked what she was most excited about and she told us she was the most excited to get home and tickle Abee:) Please pray for safe, uneventful travels and specifically for Emri's oxygen levels to stay steady on the plane! Thank you to everyone who has been lifting our family up in prayer over these last few weeks..."

October 10:

"We DID in fact make it home safely...but not without some drama of course. We got to the airport yesterday morning and made it to security. They decided to check ALL of our carry-on stuff, and we, of course, needed some special person to come look Emri over. While TSA was busy sorting through our bags, Emri was soaking her pants and creating a little puddle under her feet. When we FINALLY got clea...red, we found the only kid outfit that was in the entire airport and changed Emri. We had a great first flight to Milwaukee, and had a nice three hour layover. We boarded our next plane and once again hooked Emri up to her pulse/ox machine to monitor her oxygen level. She did great on takeoff and eventually fell asleep on daddy's lap. After about an hour into Emri's nap, I noticed her breathing harder than usual, and decided to check her oxygen again. Her oxygen was dipping into the low 80's and upper 70's. We got her oxygen machine out from under the seat but couldn't find the nasal canula that goes up Emri's nose to give her the O2. Finally after a few minutes of frantic searching, we decided to ask the flight attendant if they had one on board. Of course this set off a relatively dramatic series of events. Since Emri was still groggy and her O2 was continually dropping into the 70's, we decided to let the flight crew do whatever they needed to do to get Emri some help. The paged for a doctor or nurse on board and we got a nurse to come up an look at Emri. The nurse was concerned with Emri's dusky fingernail color and the fact that she was still groggy. The flight attendants were on the phone with whatever medical person they are supposed to call in these sort of events. FINALLY they got permission to use the oxygen tank that they had on board and let us know a few times that if her oxygen didn't go up we would have to "divert" (land at closest airport). Oh. My. Goodness. I was so nervous. Thank the LORD that as soon as Emri started receiving oxygen, her stats went right back up and her color came back. We held the mask to Emri's face until we began our descent over an hour later. As we got lower, Emri's oxygen improved on its own and by the time we landed her oxygen was back up to normal levels without oxygen. OF COURSE, its mandatory to have medics meet you on the ground...so we were escorted off the plane and Emri was checked and cleared by the medics. Right when the medics left, Brett picked up Emri only to find out she had peed through her second pair of pants for the day. Apparently she decided that yesterday was a great day to expel the 9 pounds of extra fluid on her body! SO, we got to walk through the airport...exhausted, relieved, and with our pant-less Emri.
We are home, happy, and so excited to be a family again. Thanks for the prayers, thoughts, and well wishes...we are blessed..."

Oh man do I have a long list of things I need to include in this blog....so here it goes!
I wanted to start with an update on our little big man-Abe. he is SUCH a happy, giggly, overwhelmingly busy little boy! He is crawling like a madman and beginning to test himself by letting go of furniture to balance on his feet. He is in the 95% for height and 75% for weight. He's a big kid! He eats anything and everything except for peas....HATES peas. He LOVES baths, walks, toys, remote controls, cupboards, and especially his big sister-he has the best giggles when she is entertaining him. He brings so much joy to our little family and we love him more every day!



Emri is also making some physical gains! She is now walking 6 to 10 steps with NO assistance!! We are SO proud of our little sweetheart. It is kind of sweet that Abe and Emri are learning to walk together. I can't help but think it is a sign of their relationship to come:)


There is a very exciting event coming up for our family in November. My sister, Steph, and her boy Daniel, are getting married! We are so, so excited for them, and can't wait for the wedding. Emri and Abe are flower girl and ring bearer...and mommy is nervous about how it will go...lots of bribing should get us at least a mediocre performance:)
Our house is coming along-we are now at the roofing stage of the process. After roofing, the windows will be installed, siding will be going up, and plumbing will begin. We SHOULD be able to move in sometime in November. There are SO many decisions that must be made in the coming weeks, and it is a little overwhelming to say in the least. It is such a crazy, yet exciting experience for us as a family. Brett's parents have been graciously putting up with us living in their house now for over 7 months. As much as we love spending time with Grandpa and Grandma, having live-in help with the kids, and our ongoing pinochle tournament, it will be nice for both of us to have our own space again soon. Abe can then start the demolition process on our own house instead of Grandpa and Grandmas!! ;)

October 1 is the date set for Emri's spinal rod lengthening surgery in Baltimore. We will be flying out a week early to have her plethora of appointments with the docs in Baltimore, and, Lord willing, we will be out of the hospital within a week after surgery. This IS supposed to be a less intensive surgery, but once again, every surgery for Emri is a big deal. She will, of course, need a significant amount of anesthesia, will be hooked up to a vent, and will be dependent on pain meds for a while afterwards. We have heard that although these lengthening surgeries are not as incredibly painful as the actual initial rod insertion, they are still pretty uncomfortable. Also, there is always risk of more lung issues from being put out and being on the vent. I am continually praying that God blesses our sweet girl with a quick and complication-free recovery.
We left for our trip to CA on July 26th and returned on August 6th. We were the crazy couple that decided to drive all the way there and back with a 3 year old and 9 month old. Surprisingly enough Abe was a little angel! He would make a sad little wimpery sound when he was hungry or needed his diaper changed, and slept through a majority of the trip. Emri was good too, but struggled a little to get and remain comfortable. Whoever wasn't driving was constantly re-adjusting her and stuffing blankets and towels in her car seat in an attempt to make her more comfy. She was happiest when she got to watch a movie on on the dvd player, despite the fact that it froze every 3 seconds...she didn't seem to care. I think she just felt cool because she was watching a movie in the car!
The first half of our trip was spent with family in the Escondido area. It was great to reunite with aunts, uncles, and cousins...and we even got to meet a few second cousins that we had only ever seen pictures of! 5 days of socializing, laughing, game-playing, and site-seeing later, we were headed to the marfan conference in LA.







The first day of the conference was clinic day...the day of appointment after appointment after appointment...but with doctors who know marfans...which is always quite refreshing. We first met with an eye doctor that worked at Cedars-Sinai, the hospital where the clinics were taking place. He wasn't even a bit helpful with his "not sure why you are here because we aren't going to do a full eye-exam on Emri anyway" attitude. Well, the reason we signed up for the eye clinic in the first place stemmed from our hope to see the famous Dr. Maumanee, with her strong Russian accent. This is a doctor who knows marfan eyes...which are complicated. She walked in after the first, slightly aloof Cedar-Sinai doc and did a full blown slit lamp exam on Emri. She told us that Emri needs a stronger prescription...like -25 to -30 (her RX right now is -16), and that Emri's eyes right now are about "as bad as they come." In short, Emri has thick, balled up lenses in front of her eyes that are blocking her vision. HOPEFULLY, these lenses will completely dislocate and slip down from in front of her eye. If this happens, Emri's prescription will lessen and she will be able to see much better for a long time...20-30 years before they would start agitating the eye and need to be removed. However, it could happen that the balled up lenses, in the process of falling, get stuck and can't fall all the way down...causing major irritation and glaucoma...in which case, they would have to be removed early...which vastly increases the risk of retinal detachment (blindness if not surgically corrected within 48 hours by the right doctor). Uhg...just uhg.
The next appointment was with Dr. Sponseller, Emri's orthopedic surgeon. We talked a little, but mostly just decided we would be talking in Baltimore, for Emri's lengthening surgery soon enough. Sponseller showed Emri's beautiful back off to another Cedar-Sinai Dr. and explained how he did her surgery...which I don't always enjoy listening to.
We met with Dietz next. Have I already mentioned that he is just amazing?? He read Emri's last three echo's, including one that we had gotten the day previous at Cedar. He told us that Emri's aortic root is actually more stable than the doctors at Seattle Children's were reporting it was. He was getting about the same measurement in all three echos, but said that he understood where Seattle was getting their measurement too. It all depends on your angle of measurement. We were SO happy and relieved to hear that oh-so-encouraging word...STABLE. Yes, yes yes!! It was good news, but there was a little bad news too. All three of Emri's vavles are leaking more. Her mitral valve now has moderate to severe leakage, her tricuspid is now in the moderate category, and her aortic valve has started leaking now too. Dietz told us that Emri's heart is enlarged to make up for the increased workload that comes with leaky valves, but that so far, it is handling the increased strain well. He said he will be watching her "like a hawk" and will be honest with us when he starts to get nervous. We are hoping that we can hold off on any valve surgery for a while...but know that it is most likely something we are going to have to deal with sooner than we had hoped.


Oh! Cute little story. While we were speaking with Dr. Dietz, his wonderful genetic counselor offered to play with Emri in the waiting room. When we came to get Emri after the meeting with Dietz, Gretchen told us that her and Emri had great time "grocery shopping for cheese", "celebrating Minnie's 5th birthday", and dancing. Couldn't help but laugh at not only Emri's wild imagination, but also her willingness to let Gretchen participate in it!



We also saw a Pulmonologist from Hopkins, Dr. Neptune, who has seen many, many marfan kids and adults. We didn't have many recent chest x-rays, but we agreed to meet up with her in Baltimore, after Emri's surgery, to review the x-rays that Emri will be getting during surgery of her lungs. Emphysema is a big issue in kids with severe marfans. It is devastating to see how many kids get taken because of rapid emphysema progression. Emri has lung blebs, or air pockets in the lung, which are the start of emphysema...but we are holding on to the hope that the medication she is on will halt it's progression...and looking to the future for more promising treatments. It's a hard one for me to digest...and even harder to talk about because of how many kids I have seen suffer with this horrible disease.  
The rest of the conference was informative with all the new studies coming out and fresh results from this past years' studies. We are so thankful for the people that pour their lives into helping people with marfans and other connective tissue disorders. I don't like to imagine where we would be without them. Once again, Brett and I found it hard to be in breakout sessions with parents of kids with less severe marfans. It's so hard to sit there and listen to other parents complaining about issues that are so much less life-threatening than the issues that we are dealing with in Emri. I know that to these parents, these are real and valid concerns, but it becomes easy to feel a little resentment...which I know isn't good. There are other parents at the conference with severely affected kids and it is so, so refreshing to talk with them, cry with them, and hug them. We have been forced into a friendship, but it is such a helpful and meaningful friendship. Hanging out with these wonderful moms and dads is one of the biggest reasons we go to conference. Emri had a great time connecting with other kids with marfans, and siblings without it. She was all laughs the first night we hung out with other families...Emri played the part of "tickle monster" and was "running" around on her knees trying to tickle everyone and anyone in her path. Another very important reason to go to conference...Emri making connections that will be so helpful for her...especially in the future.










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It is always so hard coming back from the conference and splashing back in to "normal" life...whatever that is. It's hard to talk about the conference because of the many intense emotional experiences that took place....not so easy to explain. It's hard to leave a place where other people "get it", and exceptionally difficult to part with families where the connections we make inevitably run so deep. At the very end of the conference, a special mom, Suely, of a child, Ethan, with severe marfans was speaking in front of the 600 conference attendees about being a mom of a kid with marfans. During her speech, Suely was receiving call after call from the nurse that was with Ethan. She finished the speech to find Ethan struggling with nausea, low oxygen, and extreme fatigue. The ambulance came to pick him up and they left to get him evaluated at Cedar-Sinai. We were so sad that we never got to formally say goodbye to them, but even more heartbroken for poor Ethan. Fortunately, everything ended up being ok...but I received a text from Suely while we were on the road heading home from the conference that said "...the life of a marfan mom!" Oh man, it's so true. One minute your heart is soaring with pride for your child who has "overcome the odds" and the next minute you are in the hospital praying that he/she does it again! It's a hard life, but it's a beautiful life. When your child is sick...perspective changes. Only the important things matter.
God is our rock through this all. There are so many times I feel so unprepared and unable to deal with all of it... but He continues to give us what we need to move forward. Because we have been faced with the reality of death, our view has and must always turn to Heaven and the promises our precious and powerful Savior has made for those who choose to believe. We could not be more thankful for this.

“What no eye has seen, what no ear has heard, and what no human mind has conceived -the things God has prepared for those who love him..."

^"My sheep listen to my voice; I know them, and they follow me. I give them eternal life, and they shall never perish; no one will snatch them out of my hand. My Father, who has given them to me, is greater than all; no one can snatch them out of my Father’s hand."

I am going to begin with Emri's latest echo results since I have not updated the blog yet on what they were. Here is my facebook post after the echo.

"One of those days that we just don't know quite how to react. Emri's aorta grew 1 mm. Doesn't seem too significant...but it is disappointing. Because the aorta is getting bigger, Emri's aortic valve is now leaking along with her mitral and tricuspid valves. Mitral valve is now in the moderate category instead of mild. Thank you for all the prayers. God is sovereign. Its just tough to look at my sweet girl and know that there is something so serious going on inside of her constantly moving little body. Next echo is in a month at the NMF conference in LA with Dietz."

So there it is. More leaking, more growing, and quite a few more tears involuntarily falling down my cheeks.
I oftentimes begin to think too much---especially after appointments like the last. What if, what if, what if?? My body begins to react in ways I don't desire it to. I start to shake, my muscles tense up, my heart pounds, I begin to breathe wrong and my fingers get tingly, my stomach starts to hurt, I feel nauseated...and on it goes."God calm me," I pray so often. "God, this is in your hands, you had this all planned out already."

Emri's physical limitations  and extra equipment (walker, feeding tube, glasses, braces) are beginning to separate her from other kids her age. Emri and I have been going to Vacation Bible School this week and it has never been more clear to me that she is now looked at as "different." She walks around either on her knees or with her walker. She has a feed going half of the morning and consequently must haul around her feeding backpack. She has been asked about her tube, her walker, why she walks on her knees, why her fingers are so long, why she can't run...etc... all in 3 days. I know right now it hurts me more than her...but I am beginning to notice her put things together. When Emri got asked why her fingers were so long I noticed her glance at her fingers...the fingers she usually holds out proudly to show off her sparkly nail polish. She put out her hand to balance on another little girls shoulder and I saw the other little girl brush Emri's hand off quickly and look at her like she had done something wrong...other kids seem to be a little frightened of Emri...just don't know what to think. I knew it would happen...but I can't even tell you how much hurt I am experiencing as her mother realizing that it's starting...she starting to understand that she isn't like other kids. I am starting to notice that when Emri feels left out or insecure she starts acting out and not listening to me. She's hurting too, and it. kills. me. 

I have often thought of this trial as one that I am meant to count as joy as it says in James 1. But lately I can't help but think of all of the instances in the Bible where someone's child is sick or dying...the parents are always in desperation mode. The love a parent has for a child is such a deep, indescribable love...when the child is hurting, sick, dying, dead...the parents hurt seems to be beyond any other kind of hurt. It's just how it seems to always go. How do I count this trial as joy when it is on my little girls' behalf?? It seems beyond impossible to me...at least right now. I know I am growing in the Lord, I am confident that I am in a better position to help, talk to, and cry with other moms in a similar situation, and I know that I am learning to give up all control that I, for some odd reason, think I still have as mine. These are all good things...all steps in the right direction...but joy?

Emri has often been described as an old soul. She has had to deal with so much already and she understands more than she should. Lately she has been crying when we have to hook up her feed and give her medicine in the morning. Brett and I have had to explain to her that if we don't give her what she needs, her heart will get too big and she would have to go back to the hospital. I have begun explaining to her that she has marfan syndrome and it makes her body weak, but that God makes her strong. When she is practicing walking, she knows that God is helping her be strong. We have been teaching her songs to sing when she feels sad or scared. We want her to know songs like "Cast all my cares", "when I am afraid", and "Thy word", so that when she is in scary situations she will have the soothing and comforting words run through her mind. These are all things I wouldn't necessarily put as a priority with a kid lacking health concerns...but with Emri, all I can do as her mother is teach her to put her trust in God know that He will never leave her.

Here it is. I am in physical, emotional and spiritual pain...I am writing now because I need to. I am so stretched thin and so exhausted--but know it is temporary. I will forever proclaim my love for the Lord and I will never blame Him or shake an angry fist at Him because I KNOW that I deserve death as a sinful human being. I can always have joy in this...that this life, here on earth, will be over in the blink of an eye. Those who are justified and sanctified through Him will be glorified in heaven....and THAT will be pure, untainted JOY. There WILL be a day of NO more tears, no more pain, and no more fears. Nothing can put a smile on my face faster than imagining Emri, with no more marfans, RUNNING as fast as she can without her braces, SEEING the face of our Lord and Savior without her glasses, and standing to worship our BEAUTIFUL and MERCIFUL Savior without her walker. And I will be there with her. THIS, people, is what it's all about.  

 

Not always good news.

So let's start with the not-so-fun news. Last Friday, April 26, Emri went in for a routine lung and heart appointment at Seattle Children's. This was the first of both checkups since spine surgery in December. Emri's lungs look and sound terrific! This is something to celebrate as terrific lungs do not always go along with having marfan syndrome, especially with Emri's severity. We were doing cartwheels in our heads hearing the doctor say that her lungs just seem to be doing excellent. Another moment worthy of celebrating came when we found out that Emri is actually ON THE WEIGHT CURVE! Emri has NEVER been on the charts for weight...ever. She has always been the little arrow at the bottom of the chart indicating that she is below the 2% mark in the weight category. Now, we do know that Emri's rods and screws in her back weigh about 10 oz, and that her clothes added a smidgen of weight also...but hey...we'll take the 7% and run with it! As for height-Emri now joins those over 100%. She is one tall, skinny glass of fun! After meeting with  the pulmonologist, we met with nutrition to discuss Emri's tube feeding, and decided to cut one tube feeding out of Emri's "feeding regimen" in order to attempt more table food consumption. This was happy news for me and especially Emri because it means more freedom from the tube she has to push or carry around while playing throughout the day...(also less "DocMcStuffins" :). After the nutrition consult we headed to cardio for Emri's echo. We are so thankful that Emri has been so cooperative for every single echo she has gone through-she just lays there, so patiently, and lets the ultra sound technician do her/his thing. Just so glad that we have not had to take part in a sedated echo up to this point. I don't know many other near 3-year-olds that would just sit there and not move for 30-40 minutes. While the echo took place I happened to look up at the screen and catch a glimpse of a number that I was so hoping was not her aorta measurement. We headed to our "favorite" room when the echo was finished-the cardio consult room. This room holds so many memories...great ones and horrifying ones. We never know what sort of memory we will be making as we walk through those all-too-familiar doors. Our cardiologist came in shortly after we got there started the small talk of how Emri was doing in all the other areas of her life. After a few minutes I finally just said, "her aorta's bigger, isn't it?" "Yes...it seems to have grown." Another moment when my heart literally starts beating out of my chest, I feel like I can't swallow properly, immediately begin to sweat...etc... I glanced over at Emri---big smile of course, flirting with the doctor...so oblivious to the fact that we are talking about her serious heart condition. I just couldn't love that girl more. She's so much braver than I will ever be.
Emri's cardiologist promised to email Dietz to get his take on everything...we exchanged pleasantries, and left. There are always tears after these kind of devastating appointments..and thoughts...so so many thoughts. Bad thoughts, terrifying thoughts, shudder-worthy thoughts...And then comes the desire to hug my sweet baby girl and NEVER, EVER let go...ever. Once again...we are ceaselessly reminded of the fragility of our precious little Emri's life. "Bless my little girl, God...bless my sweet little girl." How many times have I prayed this prayer? It's always the prayer that comes when I have no choice but to hand it all over to God. He knows the desires of my heart...He has allowed me to love this girl as much as I do...He knows. "Just bless her God."
This morning I received an email from our dearly loved Dr. Dietz. He reassured me of the fact that this jump in Emri's aortic root measurement could definitely be from the stress of spinal surgery combined with the one or two days Emri missed her medication due to blood pressure issues during and after her surgery. He did say that he has seen this before accompanying surgery. Emri's aortic root has not reached "surgery zone" measurement-wise...but it has grown very quickly in a short period of time...which is an additional reason that surgery is sometimes performed on the aortic root. Dr. Dietz ordered an increase in Emri's irbesartan dosage and a repeat echo in two months, which we already have scheduled. If you are one of Emri's many prayer warriors, please ask our merciful Savior, if it is inside His will, that we have a stable report in two months. Dr. Dietz ended his email to me with, "I remain very hopeful, and so should you." Couldn't help but smile wide...all 15 times that I read it. "Thank you Lord for this reassuring piece of hope...it's all in YOUR hands and we know that...thank you."
Abe. Abe is 6 months old today!! Such a blessing of a little dude. This kid gets to be more fun every single day. He is SUCH a sweet, loving little (or huge actually) snuggler and brings our family so much joy! His smile melts my heart...It's so amazing how much I already love this kid. He is working on sitting up, and finds his way around a room weather it be by rolling or inch-worming---he's inventive that way. I am sure sitting and crawling will be here soon, but as of now, I am enjoying every second of him still being my baby boy. He also LOVES his sister-all she has to do is knee-walk past him and he lights up. Emri is also very fond of her little brother. She enjoys advocating for Abe by telling me things like, "Mom...Abee is too yiddle to say hi to you." And she says these things so matter-of-factly. Like, "Mom...common'..he's obviously still a baby." Emri always grows very concerned when Abe is upset and lets me know that I need to feed him, or that "he must just be tired and need to sweep a yiddle bit." I am trying to soak in every moment of this stage..my babies growing up together and becoming close siblings. It's just a precious time.
Next order of business. I got a 4-hour-a-week job at Brett's place of employment. JWR Design needed some help keeping up on their new website, facebook page, and blog and thought I might be interested in helping out. It is the perfect amount of time for me to take a little break from kid world and be involved in the grown-up world.
Also, we are SO close to digging the hole to start our house! It's pretty strange that we will officially be watching walls go up so soon! We have been enjoying the process of designing our next home and feel so blessed and only slightly overwhelmed at the prospect of having to make so many decisions in a very short amount of time. If I can get myself organized enough I will be posting pictures and updating facebook and this blog as our house goes up. Our lot is pictured down below.

Last bit of fun news...Emri has officially qualified for a therapy dog! We decided to apply for a therapy dog a few months ago to help Emri not only with physical tasks (open doors, turn on lights, pick up dropped objects, help her up if she falls, pull wheelchair if needed...etc) but also for emotional support as she grows older and more aware of her disease. We are waiting to be matched with a dog-which could take a bit because we need a dog calm enough not to knock Emri over. We are ok with waiting a while since we won't even be in our house until fall. We are so excited that this opportunity came up and that Emri will get to be a part of such an amazing program. It was also interesting to find out that at the Marfan conference this year, there is a new workshop titled, "Canines can help!" Brett and I look forward to attending this workshop.
I...I think that's it for now. I hope this finds you all well...and thank you once again to all of you who take the time to keep up on this blog and to those of you praying for our family. We serve an amazing God. ~Erin~

Here are some pictures of lately...